Recurrent/Progressive Childhood Soft Tissue Sarcoma
Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials
With the possible exception of infants with congenital fibrosarcoma, the
prognosis for patients with recurrent or progressive disease is poor. The
selection of further treatment depends on many factors, including the site of
recurrence, prior therapy, and individual patient considerations. Local
recurrence or isolated pulmonary recurrence should be treated with complete
resection. All patients with recurrent tumors should be offered enrollment in
current drug studies.
Standard Treatment Options
Resection is the standard treatment for recurrent pediatric
nonrhabdomyosarcomatous soft tissue sarcomas. If the patient has not yet
received radiation therapy, adjuvant radiation should be considered after local
excision of the recurrent tumor. Limb-sparing procedures with adjuvant
brachytherapy has been evaluated in adults but has not been studied extensively
in children. For some children with extremity sarcomas who have received
previous radiation therapy, amputation may be the only therapeutic option. No
prospective trial has been able to prove that enhanced local control of
pediatric soft tissue sarcomas will ultimately improve survival. Therefore,
treatment should be individualized for the site of recurrence and
biologic characteristics (e.g., grade, invasiveness, and size) of the tumor.
All patients should be considered for enrollment in clinical trials.
Pulmonary metastasectomy may achieve prolonged disease control for some patients.[1] A large, retrospective analysis of patients with recurrent soft tissue sarcoma showed that isolated local relapse had a better prognosis and that resection of pulmonary metastases improved the probability of survival.[2]
Treatment Options Under Clinical Evaluation
Different chemotherapy regimens are currently under investigation.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood soft tissue sarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References
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Belal A, Salah E, Hajjar W, et al.: Pulmonary metastatectomy for soft tissue sarcomas: is it valuable? J Cardiovasc Surg (Torino) 42 (6): 835-40, 2001.
[PUBMED Abstract]
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Zagars GK, Ballo MT, Pisters PW, et al.: Prognostic factors for disease-specific survival after first relapse of soft-tissue sarcoma: analysis of 402 patients with disease relapse after initial conservative surgery and radiotherapy. Int J Radiat Oncol Biol Phys 57 (3): 739-47, 2003.
[PUBMED Abstract]
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